hyperammonemia

hyperammonemia

[hi″per-am″mo-ne´me-ah]

elevated levels of ammonia or its compounds in the blood. A congenital form occurs in two types: Type 1, due to deficiency of the enzyme ornithine carbamoyltransferase, is marked by vomiting, lethargy, coma, and hepatomegaly; symptoms are aggravated by protein ingestion. Type 2, due to deficiency of the enzyme carbamoyl phosphate synthetase (ammonia), is marked by vomiting, lethargy, and flaccidity and by elevated plasma and urinary levels of glycine. Hyperammonemia may also occur in nongenetic diseases such as severe liver disease.

am·mo·ne·mi·a

, ammoniemia (am'ō-nē'mē-ă, am'ō-ne-ē'mē-ă),

The presence of ammonia or some of its compounds in the blood, thought to be formed from the decomposition of urea; it usually results in subnormal temperature, weak pulse, gastroenteric symptoms, and coma.

hyperammonemia

/hy·per·am·mo·ne·mia/ (-am″o-ne´me-ah) a metabolic disturbance marked by elevated levels of ammonia in the blood.

hyperammonemia

[hī′pəram′ōnē′mē·ə]

Etymology: Gk, hyper + (ammonia), haima, blood

abnormally high levels of ammonia in the blood. Ammonia is produced in the intestine, absorbed into the blood, and detoxified in the liver. It is also generated as a by-product of protein metabolism. An increased production of ammonia or a decreased ability to detoxify it increases the blood levels of ammonia. The disorder is controlled by low-protein diets, including essential amino acid mixtures. Untreated, the condition leads to hepatic encephalopathy, characterized by asterixis, vomiting, lethargy, coma, and death.

Synlogic announced the presentation of the expanded clinical data set from its first-in-human Phase 1 study of SYNB1020, a Synthetic Biotic medicine being developed for the treatment of hyperammonemia associated with urea cycle disorders, or UCDs, and cirrhosis.

OCR-002 is an ammonia scavenger and has been granted orphan drug designation and fast track status by the US Food and Drug Administration for the treatment of hyperammonemia and resultant hepatic encephalopathy in patients with acute liver failure and acute-on-chronic liver disease.

Ocera's developmental product OCR-002, with intravenous and oral formulations, is being studied for treatment of hepatic encephalopathy, a neuropsychiatric syndrome related to hyperammonemia (excess ammonia in the blood), a complication of acute or chronic liver disease.

SYNB1020, is a novel, first-in-class, synthetic biotic medicine that is orally delivered and designed to treat elevated blood ammonia levels, or hyperammonemia, in genetic urea cycle disorders (UDDs) or in chronic liver disease.

Ocera's developmental product OCR-002, an ammonia scavenger, is being studied for treatment of hepatic encephalopathy, a neuropsychiatric syndrome associated with hyperammonemia, a complication of acute or chronic liver disease.

The neuronal dysfunction of HE is due to hyperammonemia, which is a consequence of impaired metabolic capacity of the urea cycle in the liver and intra- and extrahepatic portosystemic shunting of blood related to portal hypertension.

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